RBC Disorders Flashcards

1
Q

WHO definition of anemia

A

Hb < 13 g/dL in men

Hb < 12 g/dL in women

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2
Q

Hb and Hct are important to consider in anemic pts because certain conditions may yield low Hb levels with a normal Hct — what is an example of a condition that does this?

A

Thalassemia

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3
Q

When Hb levels do not approximate 1/3 of Hct, what are some other lab findings to check for?

A
High leukocyte counts
Lipemia
Precipitating monoclonal proteins
Cold agglutinins
Hyperglycemia
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4
Q

Normocytic anemias with primarily extravascular hemolysis

A

Hereditary spherocytosis

Sickle cell anemia

Hemoglobin C

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5
Q

Normocytic anemias with predominantly intravascular hemolysis

A

Paroxysmal nocturnal hemoglobinuria

G6PD deficiency

Immune hemolytic anemia

Microangiopathic hemolytic anemia

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6
Q

Anemias due to underproduction

A

Aplastic anemia (drugs, chemicals, viral infxn, autoimmune damage)

Myelophthisic process

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7
Q

Causes of microcytic hypochromic anemia

A

Iron deficiency

Inflammatory block to iron utilization

Thalassemia

Sideroblastic

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8
Q

Causes of macrocytic anemia

A

Stress erythropoiesis (accompanied by high retic count)

Nonmegaloblastic causes = liver disease, asplenia, hyposplenia, hypothyroid

Megaloblastic causes = B12 deficiency, folate deficiency, certain drugs, alcohol myelodysplasia

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9
Q

In what types of anemia would you see hyperchromic cells?

A

Disorders that lead to increased Hb per RBC and/or Hct, implying loss of RBC membrane in relation to RBC volume — occurs in HEMOLYTIC anemias and certain HEMOGLOBINOPATHIES

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10
Q

What lab is used to determine whether bone marrow response to anemia is adequate?

A

Reticulocyte count

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11
Q

Anemia in the setting of appropriately increased retic counts (>100,000/microliter) almost always reflect what cause(s) of the anemia?

A

Erythrocyte loss (bleeding, hemolysis)

Also occurs as a response to therapy (iron, folate, B12

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12
Q

Anemia in the setting of low retic count) indicates what cause(s) of the anemia?

A

Erythrocyte underproduction d/t deficient erythropoietin — includes the nutritional deficiencies (B12, folate, iron)

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13
Q

Leading cause, symptoms, and peripheral blood findings in aplastic anemia

A

Leading cause = autoimmune (other cause is drug-induced marrow suppression)

Symptoms occur d/t underlying pancytopenia — fatigue, dyspnea, bleeding, infection

PB shows anemia, thrombocytopenia, leukopenia

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14
Q

Management of aplastic anemia

A

Allogeneic HCT in pts <40 and otherwise healthy

Pts not eligible should receive immunosuppressive therapy with antithymocyte globulin and cyclosporin

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15
Q

PB smear findings w/iron deficiency anemia

A

Hypochromia (increased central pallor)

Anisocytosis

Poikilocytosis

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16
Q

Lab test most useful for dx of iron deficiency anemia

A

Serum ferritin

[decreased serum ferritin, increased TIBC, decreased serum iron, decreased %sat, increased FEP]

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17
Q

Tx of choice for iron deficiency anemia

A

Oral Ferrous sulfate

18
Q

Loss of vibration or position sense in the setting of macrocytic anemia might indicate _____

A

Cobalamin (B12 deficiency)

19
Q

Risk factors for folate deficiency

A

Excess alcohol use, poor diet, small-bowel disease, consider eval for celiac

20
Q

PB findings and type of anemia associated with CKD

A

Echinocytes (erythrocytes w/small number of spicules of uniform size and distribution on cell surface)

Normocytic anemia w/Low retic count

21
Q

How are ferritin, TIBC, and serum iron levels altered in anemia of chronic disease?

A

Increased ferritin
Decreased TIBC
Decreased serum iron

22
Q

_______ seen on PB smear are evidence of fragmentation hemolysis due to things like DIC, TTP, or malfunctioning heart valve

A

Schistocytes

23
Q

____ on peripheral smear often indicate immune hemolytic anemia

A

Spherocytes

24
Q

Cold agglutinin autoimmune hemolytic anemia is confirmed by what diagnostic tests?

A

Direct Coombs test positive for C3

Cold agglutinin titers

25
Q

Peripheral smear findings of target cells, teardrop cells, and/or basophilic stippling indicate what hemoglobinopathy?

A

Thalassemia

26
Q

Which of the following is true of beta-thalassemia?

A. Increased HbS
B. Increased HbA2
C. Decreased HbS
D. Decreased HbA2
E. Decreased HbF
A

B. Increased HbA2

27
Q

T/F: alpha thalassemia is not associated with diagnosis of structural Hb abnormality, but instead is diagnosed based on Hct, MCV, blood smear, and family study

A

True

28
Q

A high serum creatinine in the setting of anemia indicates what?

A

Underproduction of erythropoietin by the kidneys

29
Q

TSH is used to check for _____ which may cause anemia

A

Hypothyroidism

30
Q

Serum testosterone is used to assess for _____ in men, which may cause anemia

A

Hypotestosteronism

31
Q

Haptoglobin levels <20 mg/dL indicate _______, supported by elevated LDH and total bilirubin levels

A

Hemolysis

32
Q

Presence of urine hemosiderin and hemoglobin indicate ______

A

Intravascular hemolysis

33
Q

Precipitating factors in sickle cell anemia

A

Sickle mutation = single base change GAT—>GTT (glutamate to valine) in 6th codon of exon 1 of B-globin gene

More common in African, Hispanic, Mediterranean, Asian, and Indian descent

34
Q

Pathophysiologic process by which Hb S causes sickling

A

When Hb S becomes deoxygenated, heterotetramers polymerize to form fibrils that sickle and hemolyze

35
Q

S/s of sickle cell disease

A

Manifests at 6 months

Recurring episodes of acute pain, chronic pain, and s/s of anemia

Avg hemoglobin is 7-8 with high retic count

PE findings may include fever, tachycardia, RR 16-20, BP low normal, systolic murmur, small fibrotic spleen, leg ulcers

36
Q

Treatment for sickle cell disease

A
Hydroxyurea
ACE inhibitors
Folic acid
Pain relievers
Vaccinations to prevent infection
37
Q

Crises associated with sickle cell disease

A

Acute chest syndrome — vasoocclusion in pulmonary microcirculation; presents with chest pain, SOB, lung infiltrates, may be preceded by PNA; MCC of death in adults

Pain crises

Renal papillary necrosis — gross hematuria and proteinuria

Hepatic crisis — tender hepatomegaly

38
Q

Lab and PE findings associated with hemolytic anemia d/t plasmodium spp.

A

Parasites in RBCs on PB

Sxs of infection — fever

Splenomegaly!!

39
Q

Lab and PE findings associated with hemolytic anemia d/t Babesia microti

A

Parasites in RBCs on PB

Sxs of infection — fever

Milder malaria like illness unless pt is asplenic

40
Q

The diagnosis of __________ is confirmed by a positive osmotic fragility test and a negative direct antiglobulin (coombs) test

A

Hereditary spherocytosis

41
Q

Hereditary spherocytosis is associated with a positive family history as well as what PE and lab findings?

A

PE — splenomegaly

PB smear — spherocytes